Influence of inflammatory response, infection, and pulmonary function in cystic fibrosis

Aims: Recurrent infections and activation of the inflammatory response affect the prognosis of cystic fibrosis (CF). We investigated the relationship between inflammatory response, infection, and pulmonary function in CF. Main methods: A clinical-cross-sectional study was conducted with 86 subjects: control group (CG, n = 31, the same age and sex of the CF group), and CF group (CFG, n = 55, age: 1-16 years), further distributed into CFG negative or positive bacteriology (CFGB(-)/CFGB(+)), and CFG negative or positive Pseudomonas aeruginosa (CFGPa(-)/CFGPa(+)). Using the Wald test, multiple linear regression (95% confidence interval) was performed between CG and CFG, and between CG and each of the CF subgroups (CFGB(-)/CFGB(+) and CFGPa(-)/CFGPa(+)). The inflammatory markers evaluated were myeloperoxidase (MPO), adenosine deaminase (ADA) activities, interleukin-1beta (IL-1 beta), tumor necrosis factor-alpha (TNF-alpha), C-reactive protein (CRP), nitric oxide metabolites (NOx) levels, and total and differential leukocyte counts. Key findings: After adjusting for sex and age, CFG compared to CG revealed an increase of MPO, IL-1 beta (P < 0.001 in all subgroups), and CRP: CFG (P = 0.002), CFGB(-) (P = 0.007), CFGB(+) (P = 0.009), CFGPa(-) (P = 0.004) and CFGPa(+) (P = 0.020). NOx (P = -0.001, P < 0.001), leukocytes (P = 0.002, P = 0.001), and neutrophils (P = 0.003, P < 0.001) were increased in CFGB(+) and CFGPa(+), respectively. A negative correlation between FEV1 and leukocytes (P = 0.008) and FEV1 and neutrophils (P = 0.031) resulted in CFG. Significance: The inflammatory response characterized by the increase of MPG, IL-1 beta, and CRP is determinant for CF. Also leukocytosis due to neutrophilia determines the pulmonary function deficiency in this disease. (C) 2014 Elsevier Inc. All rights reserved.